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Kernicterus is a bilirubin-induced brain dysfunction. [1] The term was coined in 1904 by Christian Georg Schmorl . Bilirubin is a naturally occurring substance in the body of humans and many other animals, but it is neurotoxic when its concentration in the blood is too high, a condition known as hyperbilirubinemia .
Prolonged hyperbilirubinemia (severe jaundice) can result in chronic bilirubin encephalopathy (kernicterus). [5] [6] Quick and accurate treatment of neonatal jaundice helps to reduce the risk of neonates developing kernicterus. [7] Infants with kernicterus may have a fever [8] or seizures. [9] High pitched crying is an effect of kernicterus.
[citation needed] Diagnosis is usually made by investigation of a newborn baby who has developed jaundice during the first week of life. Testing. Coombs - after birth, the newborn will have a direct Coombs test run to confirm antibodies attached to the infant's red blood cells. This test is run from cord blood. [5]
Newborn infant with Rhesus disease, a type of hemolytic disease of the newborn, suffering from hydrops fetalis (edema caused by heart failure). The infant did not survive. [4] Signs of hemolytic disease of the newborn include a positive direct Coombs test (also called direct agglutination test), elevated cord bilirubin levels, and hemolytic anemia.
Kernicterus is associated with increased unconjugated bilirubin (bilirubin which is not carried by albumin). Newborns are especially vulnerable to this damage, due to increased permeability of the blood–brain barrier occurring with increased unconjugated bilirubin, simultaneous to the breakdown of fetal hemoglobin and the immaturity of gut ...
It is caused by many different diseases and agents. It is a symptom of several diseases, including GLUT1 deficiency syndrome, Lesch–Nyhan syndrome, phenylketonuria, and Huntington disease and can be a feature of kernicterus (rapidly increasing unconjugated bilirubin that cross the blood-brain-barrier in infants).
Mothers who are negative for the Kell 1 antigen develop antibodies after being exposed to red blood cells that are positive for Kell 1.Over half of the cases of hemolytic disease of the newborn owing the anti-Kell antibodies are caused by multiple blood transfusions, with the remainder due to a previous pregnancy with a Kell 1 positive baby.
Hemolytic disease of the newborn (anti-Rhc) can range from a mild to a severe disease. It is the third most common cause of severe HDN. Rh disease is the most common and hemolytic disease of the newborn (anti-Kell) is the second most common cause of severe HDN. It occurs more commonly in women who are Rh D negative.